Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity
Myelofibrosis is a rare myeloproliferative disorder. The detailed descriptions of myelofibrosis in children and adolescents is limited to a few case series and case reports describing fewer than 100 patients, thus suggesting the extreme rarity of this condition prior to adulthood. Though pediatric p...
| Main Authors: | , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-11-01
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| Series: | Frontiers in Pediatrics |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2022.1031687/full |
| _version_ | 1797984358689144832 |
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| author | Fabiola Guerra Fabiola Guerra Vincenzo L’Imperio Sonia Bonanomi Marco Spinelli Tiziana Angela Coliva Fabiola Dell’Acqua Giulia Maria Ferrari Paola Corti Adriana Balduzzi Andrea Biondi Fabio Pagni Francesco Saettini |
| author_facet | Fabiola Guerra Fabiola Guerra Vincenzo L’Imperio Sonia Bonanomi Marco Spinelli Tiziana Angela Coliva Fabiola Dell’Acqua Giulia Maria Ferrari Paola Corti Adriana Balduzzi Andrea Biondi Fabio Pagni Francesco Saettini |
| author_sort | Fabiola Guerra |
| collection | DOAJ |
| description | Myelofibrosis is a rare myeloproliferative disorder. The detailed descriptions of myelofibrosis in children and adolescents is limited to a few case series and case reports describing fewer than 100 patients, thus suggesting the extreme rarity of this condition prior to adulthood. Though pediatric patients rarely present the typical features and outcomes usually observed in older people, pediatric myelofibrosis is not considered an independent entity. Here we aim to describe patients with pediatric myelofibrosis, showing different clinical and pathological features when compared to the World Health Organization 2016 Primary Myelofibrosis classification. We retrospectively collected and analyzed 14 consecutive pediatric myelofibrosis diagnosed in our Pediatric hematology outpatient clinic over a six-year period. According to clinical data and bone marrow biopsy findings, patients were classified into three subgroups: adult-like myelofibrosis, pediatric immune myelofibrosis, idiopathic myelofibrosis. Pediatric Immune Myelofibrosis was the predominant subgroup in our cohort (7/14). Pediatric Immune Myelofibrosis is characterized by peculiar bone marrow features (i.e., T lymphocyte infiltration) and a milder course compared to the other patients Pediatric Immune Myelofibrosis is a novel and distinct pathological entity. We suggest to carefully consider Pediatric Immune Myelofibrosis in case of bone marrow biopsies showing myelofibrosis that do not fulfill WHO criteria. |
| first_indexed | 2024-04-11T07:01:15Z |
| format | Article |
| id | doaj.art-7ca332c4736d403eb287546d77247977 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-11T07:01:15Z |
| publishDate | 2022-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-7ca332c4736d403eb287546d772479772022-12-22T04:38:44ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602022-11-011010.3389/fped.2022.10316871031687Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entityFabiola Guerra0Fabiola Guerra1Vincenzo L’Imperio2Sonia Bonanomi3Marco Spinelli4Tiziana Angela Coliva5Fabiola Dell’Acqua6Giulia Maria Ferrari7Paola Corti8Adriana Balduzzi9Andrea Biondi10Fabio Pagni11Francesco Saettini12Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, ItalyTettamanti Research Center, University of Milano-Bicocca, University of Milano Bicocca, Monza, ItalyPathology, Department of Medicine and Surgery, ASST Monza, San Gerardo Hospital, University of Milano-Bicocca, Monza, ItalyPediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, ItalyPediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, ItalyPediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, ItalyPediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, ItalyPediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, ItalyPediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, ItalyPediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, ItalyDepartment of Pediatrics, University of Milano-Bicocca, European Reference Network (ERN) PaedCan, EuroBloodNet, MetabERN, Fondazione MBBM/Ospedale San Gerardo, Monza, ItalyPathology, Department of Medicine and Surgery, ASST Monza, San Gerardo Hospital, University of Milano-Bicocca, Monza, ItalyTettamanti Research Center, University of Milano-Bicocca, University of Milano Bicocca, Monza, ItalyMyelofibrosis is a rare myeloproliferative disorder. The detailed descriptions of myelofibrosis in children and adolescents is limited to a few case series and case reports describing fewer than 100 patients, thus suggesting the extreme rarity of this condition prior to adulthood. Though pediatric patients rarely present the typical features and outcomes usually observed in older people, pediatric myelofibrosis is not considered an independent entity. Here we aim to describe patients with pediatric myelofibrosis, showing different clinical and pathological features when compared to the World Health Organization 2016 Primary Myelofibrosis classification. We retrospectively collected and analyzed 14 consecutive pediatric myelofibrosis diagnosed in our Pediatric hematology outpatient clinic over a six-year period. According to clinical data and bone marrow biopsy findings, patients were classified into three subgroups: adult-like myelofibrosis, pediatric immune myelofibrosis, idiopathic myelofibrosis. Pediatric Immune Myelofibrosis was the predominant subgroup in our cohort (7/14). Pediatric Immune Myelofibrosis is characterized by peculiar bone marrow features (i.e., T lymphocyte infiltration) and a milder course compared to the other patients Pediatric Immune Myelofibrosis is a novel and distinct pathological entity. We suggest to carefully consider Pediatric Immune Myelofibrosis in case of bone marrow biopsies showing myelofibrosis that do not fulfill WHO criteria.https://www.frontiersin.org/articles/10.3389/fped.2022.1031687/fullmyelofibrosisbone marrowreticulin fibrosisautoimmune myelofibrosisinborn errors of immunitypediatric immune myelofibrosis |
| spellingShingle | Fabiola Guerra Fabiola Guerra Vincenzo L’Imperio Sonia Bonanomi Marco Spinelli Tiziana Angela Coliva Fabiola Dell’Acqua Giulia Maria Ferrari Paola Corti Adriana Balduzzi Andrea Biondi Fabio Pagni Francesco Saettini Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity Frontiers in Pediatrics myelofibrosis bone marrow reticulin fibrosis autoimmune myelofibrosis inborn errors of immunity pediatric immune myelofibrosis |
| title | Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity |
| title_full | Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity |
| title_fullStr | Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity |
| title_full_unstemmed | Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity |
| title_short | Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity |
| title_sort | pediatric immune myelofibrosis pedimf as a novel and distinct clinical pathological entity |
| topic | myelofibrosis bone marrow reticulin fibrosis autoimmune myelofibrosis inborn errors of immunity pediatric immune myelofibrosis |
| url | https://www.frontiersin.org/articles/10.3389/fped.2022.1031687/full |
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